Loeys-Dietz Syndrome and Medication

Medication is often a part of daily life for people with Loeys-Dietz syndrome (LDS). However, because of the effect LDS has on the body, not all medications are safe for people with LDS.

Keep reading to find out which medications are recommended, to be avoided, and to be used with caution in people with Loeys-Dietz syndrome:

  • Medications recommended for people with LDS: angiotensin receptor blockers (ARBs), beta-blockers, and antihistamines
  • Medications to be generally avoided in people with LDS: vasoconstrictors, stimulants, decongestants, calcium channel blockers, and fluoroquinolones
  • Medications to be used with caution in people with LDS: epinephrine injectors, bronchodilators, corticosteroids, and non-steroidal anti-inflammatory drugs (NSAIDs)

Before starting or stopping any medication, talk to your doctor (family doctor, cardiologist, allergist, pharmacist, or other specialist). It is important to discuss:

  • any illnesses you suffer from (including Loeys-Dietz syndrome)
  • any medications you are currently taking
  • recommendations regarding medications and LDS (see this page)
  • the medication you are going to start or stop, including its potential benefits and risks and instructions for its use (how to take it, how much to take and how often to take it)
Angiotensin receptor blockers (ARBs) and beta-blockers

Medication can be used to lower blood pressure and heart rate in order to reduce stress on the body’s arteries (blood vessels carrying blood from the heart to the body) and aorta (an artery attached to the heart).    

Many people with LDS take classes of medication known as angiotensin receptor blockers (ARBs) (Losartan, Candesartan, Irbesartan, etc.) and beta-blockers (Atenolol, Propanolol, Metoprolol, etc). In research studies, ARBs have been shown to decrease aneurysm growth in mice with LDS. 

During pregnancy, beta-blockers should be taken instead of angiotensin receptor blockers as ARBs can cause serious complications for the fetus. The timing for transitioning off of ARBs and on to beta blockers should be an individualized decision made with your medical team, and should occur during the preconception period or once a pregnancy test is positive.

It is important to note that beta-blockers may worsen asthma symptoms.

ARBs and beta-blockers should generally be taken at optimal titration (a personalized dose that aims to maximize the medication’s beneficial effects and minimize its side effects) and continued after vascular surgery.

Vasoconstrictors

Vasoconstrictors are a type of medication that cause the body’s blood vessels to constrict (narrow). Examples of vasoconstrictors include decongestants (they cause blood vessels in the nose and sinuses to narrow), epinephrine, norepinephrine, dopamine, and some migraine medication such as triptans.

Due to the effect of vasoconstrictors on blood vessels, people with Loeys-Dietz syndrome should avoid vasoconstrictors.

Stimulants

Stimulants are a class of drugs that boost activity in the central nervous system (brain and spinal cord). There are a variety of stimulant medications used for many reasons, including to treat ADHD. In the case of ADHD, certain stimulants help to release chemicals (norepinephrine and dopamine) in the brain and, in doing so, increase attention span and counter the effects of ADHD.

Stimulants may increase blood pressure and heart rate, and are not recommended for people with Loeys-Dietz syndrome or other conditions that affect the vascular system (Marfan syndrome, Ehlers-Danlos syndrome, and others).

Antihistamines and decongestants

Antihistamines and decongestants are two types of medication often used to treat allergy symptoms such as nasal congestion.

Antihistamines work by blocking or reducing a chemical called histamine. During an allergic reaction, the body releases histamine. This surplus of histamine in the body causes blood vessels to dilate (widen) and produces allergy symptoms such as congestion, runny nose, sneezing, eyes that are red, itchy, or watering, itchy skin, hives, and shortness of breath.

Decongestants work by constricting blood vessels throughout the body. This can help reduce congestion in the nose and sinuses but can also increase blood pressure and heart rate. Decongestants include pseudophedrine, ephedrine, phenylephrine, oxymetazoline, and naphazoline. Decongestants are present in a variety of allergy, cold, and sinus relief medications. It is important to read a medication’s list of ingredients to know whether or not it contains a decongestant.

It is recommended that people with Loeys-Dietz syndrome treat mild allergy symptoms (such as congestion) with antihistamines, and not with decongestants.

Epinephrine injectors

An epinephrine injector is about the size and shape of a marker or deck of cards and is usually injected into your thigh. It contains epinephrine (also known as adrenaline) that constricts blood vessels, increases blood pressure, increases heart rate, and expands airways to counteract severe allergic symptoms.

Because of its effect on the circulatory system, an epinephrine injector should only be used to treat anaphylactic reactions or life-threatening symptoms (not mild symptoms) in people with Loeys-Dietz syndrome. Ask your cardiologist and allergist about exactly when to use an epinephrine injector.

An allergic reaction leading to severe restrictions in the ability to breathe is referred to as anaphylaxis or an anaphylactic reaction. These reactions can be caused by foods as well as medications, dyes, and insect venom. Anaphylactic reactions can be life-threatening and should be treated immediately with an epinephrine injector and a call to emergency medical services at 911 (or other local emergency services number).

Bronchodilators and inhaled corticosteroids

Bronchodilators are medications that temporarily relax and dilate (widen) the body’s airways. They’re often used to treat asthma attacks.

As bronchodilators do not increase blood pressure, people with Loeys-Dietz syndrome should be cleared to use bronchodilators, if recommended by their doctors. Bronchodilators are able to cause local changes in the lungs, but do not result in systemic changes in the body, meaning there are no significant changes in blood pressure. You may be able to use one to treat a severe asthma attack; speak to your cardiologist and allergist about exactly when to use a bronchodilator.

Instead of a bronchodilator, your doctor may prescribe an inhaled corticosteroid to reduce inflammation and ultimately decrease the number and severity of asthma attacks you experience. Corticosteroids are not recommended in people who have uncontrolled hypertension (consistently high blood pressure) or congestive heart failure.

It is important to note that beta-blockers, a class of medication often taken by people with LDS, may worsen asthma symptoms.

Calcium channel blockers

Calcium channel blockers (CCBs) are medications used to decrease blood pressure and treat other cardiovascular problems such as angina and irregular heart rhythms. 

Based on this information, people with inherited thoracic aortic aneurysm conditions (such as Loeys-Dietz syndrome, Marfan syndrome, Ehlers-Danlos syndrome, and others) should be okay to generally take calcium channel blockers, based on their doctors’ recommendations. However, with CCBs there is potential for the medication to result in cardiac function deterioration because of negative inotropic actions, which refers to lessened contraction force in the heart.

Fluoroquinolones

Fluoroquinolones are a family of antibiotics. They include Factive, Cipro, Avelox, Levaquin, and Floxin.

Fluoroquinolones use should be avoided in people with Loeys-Dietz syndrome or other conditions that increase the risk of aortic aneurysm or dissection, such as Marfan syndrome or Ehlers-Danlos syndrome.

A December 2018 warning from the FDA explains that medical professionals should avoid fluoroquinolones use in these patients, unless there is no alternative antibiotic that would be effective in the treatment of a serious infection.

This warning is based on cases reported to the FDA as well as four observational studies. Together, the studies show that individuals taking fluoroquinolones had approximately twice the risk of experiencing an aortic dissection or aortic aneurysm rupture.

If a prescription for fluoroquinolones must be made, medical professionals and patients should discuss the risks and symptoms of aortic dissection, and emphasize that a person experiencing symptoms of aortic dissection must immediately seek emergency services by calling 911 or going to an emergency room.

Non-steroidal anti-inflammatory drugs (NSAIDs)

Non-steroidal anti-inflammatory drugs (NSAIDs) can help reduce fever, inflammation, and pain by blocking the body’s production of chemicals called prostaglandins. NSAIDs include aspirin, ibuprofen, naproxen, diclofenac, and celecoxib, and can also be found in other cold, flu, and pain-relief medication.

It is recommended that NSAIDs are used with caution in people with high blood pressure and in people taking medication to lower their blood pressure. Research has found that use of NSAIDs can increase blood pressure an average of 3-5 mmHg.

People with Loeys-Dietz syndrome may take medication to lower their blood pressure. These medications include angiotensin receptor blockers (ARBs) (Losartan, Candesartan, Irbesartan, etc.) and beta-blockers (Atenolol, Propanolol, Metoprolol, etc). Since NSAIDs can raise blood pressure, they may reduce the effectiveness of ARBs or beta-blockers.

Talk to your medical professional about if and/or when taking NSAIDs along with an angiotensin receptor blocker or beta-blocker is recommended for you.

It is recommended that people taking blood thinners (anticoagulants and antiplatelets) avoid or use caution when taking NSAIDs. NSAIDs can prevent or slow blood clotting and increase the risk of bleeding, especially when taken with a blood thinner. If it is necessary to take both, your medical professional should review precautions and monitoring options with you.

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